Dataset Information

Pontomesencephalic Atrophy and Postural Instability in Wilson Disease.

ABSTRACT:

Background and purpose

The MR Parkinsonism index helps in differentiating progressive supranuclear palsy from Parkinson disease and multisystem atrophy. Pontomesencephalic involvement is common in neurologic Wilson disease, but there is no prior study evaluating the MR Parkinsonism index and its indices in Wilson disease. We report the MR Parkinsonism index and its indices in Wilson disease and correlate these changes with clinical severity and postural reflex.

Materials and methods

Thirteen individuals with neurologic Wilson disease were included, and their clinical details, including neurologic severity, postural reflex abnormality, and location of signal changes on MR imaging, were noted. The 3D BRAVO T1 sequence was used for measurement of the MR Parkinsonism index and its indices. The MR Parkinsonism index and its indices were also obtained in 6 age- and sex-matched controls. The morphometric parameters in Wilson disease were compared with those in with healthy controls and among the patients with and without abnormal postural reflex.

Results

The midbrain area was reduced in patients with Wilson disease compared with controls (112.08 ± 27.94 versus 171.95 ± 23.66 mm², P = .002). The patients with an abnormal postural reflex had an increased MR Parkinsonism index and pons-to-midbrain ratio compared with controls, whereas these parameters were equivalent in patients with normal postural reflex and controls. The patients with abnormal postural reflex had more severe illness, evidenced by higher Burke-Fahn-Marsden scores (51.0 ± 32.27 versus 13.75 ± 12.37, P = .04) and neurologic severity grades (2.57 ± 0.53 versus 1.67 ± 0.82, P = .04).

Conclusions

An increase in the MR Parkinsonism index in Wilson disease is mainly due to midbrain atrophy and it correlates with neurologic severity and abnormal postural reflex.

SUBMITTER: Kalita J

PROVIDER: S-EPMC7959919 | biostudies-literature | 2017 Jul

REPOSITORIES: biostudies-literature

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Publications

Pontomesencephalic Atrophy and Postural Instability in Wilson Disease.

Kalita J J Naik S S Bhoi S K SK Misra U K UK Ranjan A A Kumar S S

AJNR. American journal of neuroradiology 20170511 7

<h4>Background and purpose</h4>The MR Parkinsonism index helps in differentiating progressive supranuclear palsy from Parkinson disease and multisystem atrophy. Pontomesencephalic involvement is common in neurologic Wilson disease, but there is no prior study evaluating the MR Parkinsonism index and its indices in Wilson disease. We report the MR Parkinsonism index and its indices in Wilson disease and correlate these changes with clinical severity and postural reflex.<h4>Materials and methods</ ...[more]

PMID: 28495941

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Project description:Background: Postural instability is a debilitating and largely treatment-resistant symptom of Parkinson's disease (PD). A better understanding of the neural substrates contributing to postural instability could lead to new targets for improved pharmacological and neurosurgical interventions. However, investigating these neural substrates necessitates the use of functional MRI scanners, which are almost exclusively horizontally-based. Objective: We aimed to develop, and validate the use of, an MRI compatible balance simulator to study static and dynamic balance control in PD patients and elderly controls. Methods: Our MRI compatible balance simulator allowed participants to actively balance an inverted pendulum by activating postural muscles around the ankle joint while supine. Two studies were performed to compare static and dynamic balance performance between upright stance and simulated stance in PD patients and controls. Study 1 (14 PD; 20 controls) required participants to maintain static balance during upright and simulated stance for 120 s with eyes open and closed. In study 2 (20 PD; 22 controls) participants repeated the static balance task (80 s, eyes closed only), and also completed a dynamic balance task which required maintaining balance while experiencing random anterior-posterior perturbations applied to the trunk/pendulum. Postural sway of the body/pendulum was measured using an angular velocity sensor (SwayStarTM, study 1) and Optotrak motion capture (study 2). Outcome measures were amplitude and frequency of center of mass sway for static balance, and peak and time-to-peak of center of mass displacement and velocity for dynamic balance. Results: PD patients had larger sway amplitude during both upright and simulated static balance compared to controls. PD patients had larger peak and time-to-peak sway, and larger time-to-peak sway velocity, during simulated, but not upright, dynamic balance compared to controls. Conclusions: Deficits in static and dynamic balance control can be detected in PD patients using a novel MRI compatible balance simulator. This technique allows for functional neuroimaging to be combined with balance-relevant tasks, and provides a new means to create insights into the neural substrates contributing to postural instability in PD.

Project description:BackgroundVisual dysfunction, including abnormal stereopsis, is a significant non-motor symptom in Parkinson's disease (PD) that can reduce quality of life and appears early in the disease. Abnormal stereopsis is associated with worsening of bradykinesia and freezing of gait, though the exact pathways linking stereopsis to motor symptoms remain unclear. Furthermore, in PD patients, the pedunculopontine nucleus and laterodorsal tegmental complex play an active role in sensorimotor control, and these areas provide cholinergic projections. Cholinergic degeneration may be associated with symptoms such as abnormal stereopsis, postural instability, gait disturbances and cognitive impairment. Therefore, in this study, we hypothesized that a high postural instability and gait disturbance score would increase the risk of abnormal stereopsis in PD.MethodsWe designed a cross-sectional study and included 240 early PD patients without ophthalmologic problems other than abnormal stereopsis. To evaluate stereopsis, we used Titmus stereo test plates. Stereopsis testing was performed only once at the time of the patient's initial PD diagnosis. We collected data from medical history taking, scales, cognitive function tests, gait analysis, and tilt table tests. To analyze the potential risk factors for abnormal stereopsis in PD, we conducted a binary logistic stepwise selection analysis.ResultsAmong the total of 240 PD patients, 185 were in the normal stereopsis group and 55 were in the abnormal stereopsis group. In the analysis for risk factors related to abnormal stereopsis, the postural instability and gait difficulties (PIGD) subtype score was significantly associated with abnormal stereopsis. (95% confidence interval: 1.37-5.15).ConclusionsIn PD, particularly in PIGD subtype patients, abnormal stereopsis can lead to a decrease in the quality of sensory information, potentially interfering with feedback and adaptation processes. This, in turn, can negatively affect posture and gait, creating a vicious cycle.

Dataset Information

Pontomesencephalic Atrophy and Postural Instability in Wilson Disease.

Background and purpose

Materials and methods

Results

Conclusions

Publications

Pontomesencephalic Atrophy and Postural Instability in Wilson Disease.

Similar Datasets

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